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MSK > Systemic Autoimmune Diseases > Flashcards

Systemic Autoimmune Diseases Flashcards

1
Q

What are the major CT disease?

A 
SLE
Scleroderma
Sjogren's Syndrome
Auto-immune myositis
Mixed CT disease e.g. Raynauds, soft tissue swelling
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2
Q

What are the major Systmic Vasculitides

A

GCA
GPA (Wegener’s)
Microscopic Polyangiitis
Churg-Strauss

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3
Q

Who gets SLE?

A

Women mostly
15-50yrs

More common in afro-caribbeans > Asians > caucasian

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4
Q

What criteria are needed to diagnose SLE?

A

4 or more of:

  • Malar Rash
  • Discoid rash
  • Photosensitivity
  • Oral ulcers
  • Arthritis (at least 2 joints)
  • Serositis (pleurisy or pericarditis)
  • Renal (proteinuria or cellular casts in urine)
  • Neuro (seizures or psychosis)
  • Haemotology (Low WCC, platelets, lymphocytes or haemolytic anaemia)
  • Immunological
  • ANA: autoimmune antibodies
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5
Q

What is included in the “immunological” criteria for SLE?

A 
Anti ds-DNA Abs
Anti-SM Abs
Anti Cardiolipin Abs
Lupus Anticoagulant
Low complement
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6
Q

Other than the primary criteria, what other symptoms/signs can come from SLE?

A 
Alopecia
Fever
Depression
 Vasculitis, purpura & Urticaria
Pleural effusion & Pulm fibrosis
Raynaud's
Aseptic necrosis of hip
Myopathy
Abdo Pain
Aortic Valve Lesions
Cranial nerve lesions, peripheral neuropathy and ataxia
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7
Q

Who gets Scleroderma?

A

Mostly female

Onset 30-50yrs

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8
Q

What are the types of scleroderma?

A

Localised (aka Morphea OR Linear) : only the skin is affected

Generalised (limited or Diffuse): internal organs are also affected.

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9
Q

What does localised scleroderma look like?

A

localised patches of hardened skin that are smooth and shiny.

Usually on the trunk
Painless

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10
Q

What does limited SCleroderma look like?

A

Thickening of skin and colour change in face and extremities along with Raynaud’s

CAn progress to affect gut –> heartburn & difficulty swallowing

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11
Q

What do you see in diffuse scleroderma?

A

Affects the whole body, with potential to hit the heart, lungs and kidneys

Expect fatigue, joint pain & stiffness

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12
Q

What is the general presentation of Scleroderma?

A 
hardening of the skin
swelling of the hands and feet
joint pain
stiffness
Raynauds' Syndrome.

All due to excess collagen

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13
Q

What are the major complications of scleroderma?

A

Limited –> Pulm Hypertension

Diffuse –> Pulm fibrosis, renal crisis and small bowel bacterial overgrowth

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14
Q

Who gets Sjogren’s Syndrome?

A

Mostly women

40-50yrs

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15
Q

What’s the classic presentation of Sjogren’s Syndrome?

A

Dry eyes & mouth
Parotid Enlargement
~Systemic upset incl fever, fatigue, myalgia &arthralgia

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16
Q

Complications of Sjogren’s Syndrome?

A 
Lymphoma
Neuropathies (Cranial nerve, peripheral, fits etc)
Purpura
Interstitial Lung Disease
Renal Tubular Acidosis
17
Q

Auto-immune myositis is very rare. how does it present?

A

Symmetrical diffuse proximal muscle weakness.

Can be polymyositis or dermatomyositis

Dermatomyositis can come with Gottron’s papules (hands) and heliotrope rash
(face)

18
Q

GCA affects the large arteries, what do you need to diagnose it?

A

3+ of:

  • Age >50yrs at onset
  • New headache
  • Temporary art tenderness
  • ESR >50
  • Abnormal temporal biopsy
19
Q

Which conditions are Anca associated Vasculitis?

A

Wegeners (GPA = granulomatosis with polyangiitis
Micrsocopic Polyangiitis
Eosinophilic Granulomatosis with polyangiitis

20
Q

What happens in GPA?

A

Necrotising granulomatous INFLAMMATION

Occurs mainly in Upper & Lower resp tract in small and medium vessels along with necrotising glomerulonephritis

21
Q

What’s different about MPA from GPA?

A

MPA doesn’t include immune deposites or granulomatous inflammation

Instead you get necrotising VASCULITIS of small vessels and necrotising glomerulonephritis. Sometimes with pulmonary capillaritis

22
Q

What is Churg Strauss?

A

Eosinophilic Granulomatosis with Polyangiitis (EGPA)

Eosinophil rich necrotising granulomatous INFLAMMATION of small to med vessles

Mostly resp tract

Ass with asthma and eosinophilia

23
Q

AAVs can lead to what kind of complications?

A 
Hearing loss
Renal failure
Lung damage
Malignancy
Nerve damage
24
Q

What ANA profiles are targeted in SLE?

A

dsDNA
Ro
Sm

25
Q

What ANA profiles are targeted in Scleroderma?

A

Scl-70

Centromere

26
Q

What ANA profiles are targeted in polymyositis?

A

Jo-1

27
Q

What ANA profiles are targeted in Sjogren’s disease?

A

Ro

La

28
Q

What other tests are relevant to these conditions?

A 
FBC
U&Es + Urinalysis
CXR
ECG
ESR/CRP
aPTT

Renal biopsy for nephritis

29
Q

What are the stages of Lupus Nephritis?

A 
1 - minimal mesangial
2 - mesangial proliferative
3 - focal
4 - diffuse
5 - membranous
6 - advanced sclerosing
30
Q

How do we treat these conditions?

A

Mild - Hydroxychloroquine
Mod - Azathioprine, MTX or Mycophenolate
Severe - Cyclophosphomide or Rituximab

CCS can be used an adjunct or for specific symptoms but are not general 1st line treatments

31
Q

Which antibodies are tested for in SLE patients who are pregnant and why?

A

Anti-Ro
Anti-La

They are both indicative of a high risk of neonatal Lupus

32
Q

Try using this mnemonic to remember all the criteria for SLE: Dopamine Rash

A 
Discoid Rash
Oral Ulcer
Photosensitvity
Arthritis
Malar Rash
Immunological (Anti-sm, anti-dsDNA, Anti-phospholipid, anti-ro ; anti-la)
Neuro symptoms (Psychosis, seizure, personality)
ESR (raised)

Renal (Proteinuria or cellular casts)
ANA
Serositis (Pleurisy &Pericarditis)
Haematology (haemolytic anaemia, thrombocytopenia, leucopenia)

33
Q

Pathogenesis of SLE?

A

Autoantibodies are formed against a subtype of autoantigens (ANA). This leads to the development of immune complexes. The inability to clear these complexes leads to hosts immune response.

MSK (40 decks)

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